Nefropatías congénitas y hereditarias (I)tubulopatías
- Gili, B. Quiroga
- del Valle, K.M. Pérez
- Sevillano, B. Hernández
- de la Fuente, G. de Arriba
ISSN: 0304-5412
Year of publication: 2019
Issue Title: Enfermedades nefrourinarias (V)Enfermedades renales tubulointersticiales.Nefropatías hereditarias y congénitas
Series: 12
Issue: 83
Pages: 4878-4884
Type: Article
More publications in: Medicine: Programa de Formación Médica Continuada Acreditado
Abstract
Tubulopathies comprise a set of inherited or acquired renal disorders presenting ionic abnormalities. According with renal tubule segment involved, tubulopathies are classified in: impairment of proximal tubule (glucosuria, aminoaciduria, phosphaturia, hypouricemia, proximal tubular acidosis type II or Fanconi syndrome which encompasses most of them in a complex way); impairment of Henle's loop and distal tubule (Bartter syndrome, Gitelman syndrome and magnesium depletion); impairment of collecting tubule (Liddle syndrome, tubular acidosis I and IV, and nephrogenic diabetes insipidus). Overall, prognosis is favorable in the most of cases. Treatment requires fluid and electrolyte replacement.
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